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Ann Thorac Surg 2003;75:323
© 2003 The Society of Thoracic Surgeons
a Pediatric Cardiac Surgery, The University of Chicago Children’s Hospital, 5841 S Maryland Ave, MC 5040, Chicago, IL 60637, USA
To the Editor:
We read with great interest the case report by Tchervenkov and collegues [1] describing the successful repair of the anatomic constellation of complete atrioventricular canal (CAVC), double-outlet right ventricle (DORV), cor triatriatum, and left (SVC) to unroofed coronary sinus (CS). We recently had the opportunity to operate on a patient with identical anatomy. Because of the rarity of such an association, we would like to present our patient and comment on a few points.
A 9-month-old child was referred to us with a diagnosis of CAVC, DORV, pulmonary and infundibular stenosis, cor triatriatum, and left SVC to unroofed CS. She was mildly cyanotic (saturation 92% on room air). At operation, in addition to the previously mentioned anatomic features, the ventricular septal defect (VSD) was strictly in the inlet portion of the septum, and the aorta arose entirely from the right ventricle. There was a double conus. There were no atrioventricular valve attachments to the anterior crest of the VSD. The pulmonary annulus z score was -1 with a tight infundibulum. Repair was undertaken on cardiopulmonary bypass (CPB) and hypothermia to 25°C. A right atriotomy and infundibulotomy were performed. The VSD was enlarged supero-anteriorly first, resecting a generous portion of interventricular septum and conal septum. The VSD was then closed with a baffle created out of two autologous pericardial patches angled at about 45° [2]. The CAVC repair was then completed in standard fashion. A pulmonary valvotomy was performed, and the infundibulum was patched with pericardium. The left atrial membrane was resected. The SVC ostium was intimately involved with the left upper pulmonary vein ostium. An intracardiac baffle would have caused pulmonary vein stenosis, therefore an extracardiac reconstruction was chosen. The left SVC was anastomosed to the right atrial appendage using a flap of atrial tissue posteriorly and autologous pericardium anteriorly. The patient was weaned from CPB in sinus rhythm. The aortic cross-clamp time was 190 minutes and the CPB time was 325 minutes. She was discharged from the hospital on postoperative day 10 in good condition. Echocardiogram showed mild mitral regurgitation, a wide open left ventricular outflow tract, and a mean gradient of 15 mm Hg across the right ventricular outflow (RVOT). At a 4 month follow-up she remains well and her echocardiogram is unchanged.
Apart from the striking similarity between these two very rare cases, several technical aspects differ. A conduit was not used to reconstruct the RVOT, mainly because of the combination of mild valvar pulmonary stenosis (PS) with severe subPS, allowing us to preserve the pulmonary valve. Extracardiac reconstruction of the LSVC was chosen because of the proximity of the left-sided upper pulmonary vein.
In closing, we prefer the use of the term VSD extension rather than VSD translocation. The VSD is really more extended or enlarged supero-anteriorly rather than translocated, which implies closure of the existing VSD and the creation of a new VSD with no connection to the previous one.
We congratulate Dr Tchervenkov and collegues [1] for an excellent outcome.
References
This article has been cited by other articles:
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  J. H. Shuhaiber, S. Y. Ho, M. Rigby, and B. Sethia Current options and outcomes for the management of atrioventricular septal defect Eur. J. Cardiothorac. Surg., May 1, 2009; 35(5): 891 - 900. [Abstract] [Full Text] [PDF]
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